A5006444957- Alkaline Phosphatase Research Studies
- Heterotopic Ossification and Related Conditions
- Biochemical and Molecular Research
- Osteomyelitis and Bone Disorders Research
- Bone health and treatments
- Bone health and osteoporosis research
- Orthopedic Infections and Treatments
- Blood disorders and treatments
- Vitamin D Research Studies
- thermodynamics and calorimetric analyses
- Immunodeficiency and Autoimmune Disorders
- Bone and Joint Diseases
- Autoimmune and Inflammatory Disorders Research
- Asymmetric Synthesis and Catalysis
- Immune Cell Function and Interaction
- Blood groups and transfusion
- Selenium in Biological Systems
- Synthesis and Catalytic Reactions
- Chemical Synthesis and Analysis
- Hematological disorders and diagnostics
- Oxidative Organic Chemistry Reactions
- T-cell and B-cell Immunology
- Musculoskeletal Disorders and Rehabilitation
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
- Vector-borne infectious diseases
Universitätsklinikum Würzburg
2013-2025
Universitäts-Kinderklinik Würzburg
2013-2023
Society of Paediatric Oncology and Haematology
2023
University of Würzburg
2012-2021
Justus-Liebig-Universität Gießen
1999-2021
Bayer (Germany)
2018
Children's Hospital
2017
Giessen School of Theology
2013-2015
Klinikum Ludwigsburg
2015
University Children's Hospital Tübingen
2015
Hypophosphatasia (HPP) is an inborn error of metabolism that, in its most severe perinatal and infantile forms, results 50-100% mortality, typically from respiratory complications.Our objective was to better understand the effect treatment with asfotase alfa, a first-in-class enzyme replacement therapy, on mortality neonates infants HPP.Data patients forms HPP two ongoing, multicenter, multinational, open-label, phase 2 interventional studies alfa were compared data similar retrospective...
An electrochemical model is derived for the charging of Li–O<sub>2</sub> batteries with dissolved redox mediators and applied to selected nitroxides.
BACKGROUND. Hypophosphatasia (HPP) is a rare genetic disorder resulting in variable alterations of bone formation and mineralization that are caused by mutations the ALPL gene, encoding tissue-nonspecific alkaline phosphatase (ALP) enzyme.
Long-term data on enzyme replacement treatment of hypophosphatasia (HPP) are limited. To evaluate efficacy and safety asfotase alfa in patients aged ≤5 years with HPP followed for up to 6 years. Phase 2 open-label study (July 2010 September 2016). Twenty-two sites; 12 countries. Sixty-nine [median (range) age: 16.0 (0.02 72) months] severe sign/symptom onset before age months. Asfotase mg/kg three times/week or 1 six subcutaneously. Primary measure: Radiographic Global Impression Change...
Abstract Here we report the development of first enantioselective Dakin–West reaction, yielding α‐acetamido methylketones with up to 58 % ee good yields. Two obtained products were recrystallized once achieve 84 . The employed methylimidazole‐containing oligopeptides catalyze both acetylation azlactone intermediate and terminal decarboxylative protonation. We propose a dispersion‐controlled reaction path that determines asymmetric reprotonation enolate after decarboxylation.
Objective Antinuclear antibody (ANA)–positive juvenile idiopathic arthritis (JIA) is characterized by synovial B cell hyperactivity, but the precise role of CD4+ T cells in promoting local activation unknown. This study was undertaken to determine phenotype and function that promote aberrant JIA. Methods Flow cytometry performed compare cytokine patterns PD‐1 high fluid (SF) patients with JIA follicular helper tonsils control individuals. TCRVB next‐generation sequencing used analyze subsets...
The human granulocyte alloantigen NB1, recently clustered as CD177, is heterogenously expressed on neutrophils of 88-97% healthy individuals. Since its molecular nature has remained unknown, we isolated NB1 glycoprotein from lysate by immunoaffinity chromatography. MALDI-TOF mass spectrometry identified a 50,556 Da which was reduced to 43,069 after removal N-linked carbohydrates. Following N-terminal amino acid sequencing and NB1-specific primer construction, rapid amplification cDNA ends...
Abstract Background Hypophosphatasia (HPP) is a rare, inherited metabolic disorder caused by loss-of-function mutations in the ALPL gene that encodes tissue-nonspecific alkaline phosphatase TNAP (ORPHA 436). Its clinical presentation highly heterogeneous with remarkably wide-ranging severity. HPP affects patients of all ages. In children HPP-related musculoskeletal symptoms may mimic rheumatologic conditions and diagnosis often difficult delayed. To improve understanding order to shorten...
Objective : Studies of outcome in patients with medullary thyroid carcinoma require long follow-ups to accrue enough cancer-specific events. Owing time-dependent changes diagnosis and therapy, observation periods render studies susceptible ‘time bias’ which can yield spurious results. This study was designed investigate event rates after initial neck resection contemporaneous recruited within less than a decade. Design Institutional cohort 128 consecutive who underwent compartment-oriented...
BACKGROUNDAntibiotic-Refractory Lyme Arthritis (ARLA) involves a complex interplay of T cell responses targeting Borrelia burgdorferi antigens progressing toward autoantigens by epitope spreading. However, the precise molecular mechanisms driving pathogenic response in ARLA remain unclear. Our aim was to elucidate program disease-specific Th cells.METHODSUsing flow cytometry, high-throughput receptor (TCR) sequencing, and scRNA-Seq CD4+ cells isolated from joints patients with living Europe,...
The granulocyte antigens HNA‐1a, ‐1b, and ‐1c (formerly named NA1, NA2 SH) which reside on the neutrophil FcγReceptor IIIb (FcγRIIIb) play a major role in immune neutropenias pulmonary transfusion reactions. In an attempt to shed some light origin history of these we typed DNA Blacks from South Africa ( n =99), Ghana =27), 56 African Americans, 138 Chinese Taiwan for HNA‐1a,‐1b, using polymerase chain reaction with sequence‐specific primers (PCR‐SSP). American Blacks, HNA‐1b antigen was more...
summary The aim of this study was to investigate which approach for serological testing multiparous donors might be feasible and effective reduce the risk transfusion‐related acute lung injury (TRALI). TRALI is a serious adverse event blood transfusion. Antibodies granulocytes human leucocyte antigens (HLAs) are frequently detected in sera implicated donors. These often women. A general deferral female plasma or screening strategies antibodies has been proposed increase safety. prospective...